Primary Extra-axial Glioblastoma: Case Report and Literature Review

Glioblastoma multiforme (GBM) is the most frequent and most aggressive primary brain tumor in adults,mainly located in the cerebral hemispheres. In the literature, few cases of primary GBM have been reported to have radiographic and intraoperative features of extra-axial lesions, leading to a diagnostic dilemma. Despite the advances in imaging modalities, the diagnosis of GBM can be challenging, and it is mainly based on the histopathologic confirmation of the excised tumor. We describe the case of a 76- year-old previously healthy female patient who presented to our hospital due to speech disturbances and cognitive impairment. The diagnosis of the tumor type on magnetic resonance imaging (MRI) was difficult, as the findings were suggestive of a malignant meningioma due to the heterogeneous enhancement of a dural-based mass with a dural tail sign. Moreover, the intraoperative findings revealed an extra-axial mass attached to the dura. A histological examination confirmed the diagnosis of glioblastoma with arachnoid infiltration. The patient underwent adjuvant radiotherapy and concomitant temozolomide treatment, she had clinical improvement postoperatively, and was stable during the six months of follow-up. Glioblastoma should be considered in the differential diagnosis of primary extra-axial mass with atypical and malignant features, especially in elderly patients.

Spontaneous Disappearance of an Arachnoid Cyst after Burr Hole Drainage of Chronic Subdural Hematoma / 대한신경손상학회지

It is well known that the presence of arachnoid cysts (ACs) in young patients is a risk factor for developing a chronic subdural hematoma (CSDH) after a minor head injury. Although there have been controversies with the treatment, most authors recommend only draining the CSDH if the AC is asymptomatic. This judgement is based on the facts that this surgical approach has shown good clinical outcomes, and the AC usually remains unchanged after the surgery. Our case demonstrates that the AC of a young patient who developed a CSDH after a minor head injury completely disappeared after a burr hole drainage of the CSDH. Although the chances of an AC disappearing are low, this case shows that an AC might disappear after only draining a CSDH when a rupture of the AC membrane is identified. In such cases, we recommend first draining only the CSDH for the treatment of AC-associated CSDHs.

Disappearance of Arachnoid Cyst after Burrhole Trephination: Case Series / 대한신경손상학회지

We report 3 cases of arachnoid cysts (ACs) that completely disappeared after burr hole drainage, without cyst fenestration into the subarachnoid space or cystoperitoneal shunt. The first patient was a 21-year-old female with an AC of the right cerebral convexity, found incidentally. After endoscopic AC fenestration was performed, the patient complained of persistent headache. Two-month postoperative brain imaging revealed reaccumulated AC and associated multi-stage subdural hematoma. Burr hole drainage was performed to resolve the chronic subdural hematoma (CSDH). Three months later, brain computed tomography showed that the CSDH and the AC had disappeared. The second patient was an 11-year-old male who had a history of trauma 1 month prior to presentation at the clinic. Brain magnetic resonance imaging revealed an AC in the left sylvian fissure with CSDH. We performed burr hole drainage to treat the CSDH first. Subsequently, the AC as well as the CSDH disappeared. The third case was an AC of the right parietal convexity, found incidentally. Only burr hole drainage was performed, following which, the AC disappeared. This case series shows that an AC can disappear naturally after rupture into the subdural space by trauma or the burr hole procedure.

Cervical Cerebrospinal Fluid Leakage Concomitant with a Thoracic Spinal Intradural Arachnoid Cyst / 대한신경손상학회지

We encountered a very rare case of spontaneous spinal cerebrospinal fluid (CSF) leakage and a spinal intradural arachnoid cyst (AC) that were diagnosed at different sites in the same patient. These two lesions were thought to have interfered with the disease onset and deterioration. A 30-year-old man presented with sudden neck pain and orthostatic headache. Diplopia, ophthalmic pain, and headache deteriorated. CSF leakage was confirmed in C2 by radioisotope cisternography, and an epidural blood patch was performed. While his symptoms improved gradually, paraparesis suddenly progressed. Thoracolumbar magnetic resonance imaging (MRI) revealed an upper thoracic spinal intradural AC, which was compressing the spinal cord. We removed the outer membrane of the AC and performed fenestration of the inner membrane after T3-4 laminectomy. Postoperative MRI showed complete removal of the AC and normalized lumbar subarachnoid space. All neurological deficits including motor weakness, sensory impairment, and voiding function improved to normal. We present a case of spontaneous spinal CSF leakage and consecutive intracranial hypotension in a patient with a spinal AC. Our report suggests that if spinal CSF leakage and a spinal AC are diagnosed in one patient, even if they are located at different sites, they may affect disease progression and aggravation.

Comparison among ACR1997, SLICC and the new EULAR/ACR classification criteria in childhood-onset systemic lupus erythematosus

Abstract Background: To date there are no specific classification criteria for childhood-onset systemic lupus erythematosus (cSLE). This study aims to compare the performance among the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics criteria (SLICC) and the new European League Against Rheumatism (EULAR)/ACR criteria, in a cSLE cohort. Methods: We conducted a medical chart review study of cSLE cases and controls with defined rheumatic diseases, both ANA positive, to establish each ACR1997, SLICC and EULAR/ACR criterion fulfilled, at first visit and 1-year-follow-up. Results: Study population included 122 cSLE cases and 89 controls. At first visit, SLICC criteria had higher sensitivity than ACR 1997 (89.3% versus 70.5%, p < 0.001), but similar specificity (80.9% versus 83.2%, p = 0.791), however performance was not statistically different at 1-year-follow-up. SLICC better scored in specificity compared to EULAR/ACR score ≥ 10 at first visit (80.9% versus 67.4%, p = 0.008) and at 1-year (76.4% versus 58.4%, p = 0.001), although sensitivities were similar. EULAR/ACR criteria score ≥ 10 exhibited higher sensitivity than ACR 1997 (87.7% versus 70.5%, p < 0.001) at first visit, but comparable at 1-year, whereas specificity was lower at first visit (67.4% versus 83.2%, p = 0.004) and 1-year (58.4% versus 76.4%, p = 0.002). A EULAR/ACR score ≥ 13 against a score ≥ 10, resulted in higher specificity, positive predictive value, and cut-off point accuracy. Compared to SLICC, a EULAR/ACR score ≥ 13 resulted in lower sensitivity at first visit (76.2% versus 89.3%, p < 0.001) and 1-year (91% versus 97.5%, p = 0.008), but similar specificities at both assessments. When compared to ACR 1997, a EULAR/ACR total score ≥ 13, resulted in no differences in sensitivity and specificity at both observation periods. Conclusions: In this cSLE population, SLICC criteria better scored at first visit and 1-year-follow-up. The adoption of a EULAR/ACR total score ≥ 13 in this study, against the initially proposed ≥10 score, was most appropriate to classify cSLE. Further studies are necessary to address if SLICC criteria might allow fulfillment of cSLE classification earlier in disease course and may be more inclusive of cSLE subjects for clinical studies.

Cauda Equina Syndrome Occurred by Adhesive Arachnoiditis of the Lumbar Spine with an Unknown Cause / 대한정형외과학회잡지

Spinal adhesive arachnoiditis is an inflammation and fibrosis of the subarachnoid space and pia mater caused by infection, trauma, spinal vascular anomalies, and iatrogenic (surgery and/or puncture). Adhesive arachnoiditis develops various symptoms and signs (gait disturbances, radiating pain, paralysis, and incontinence). On the other hand, adhesive arachnoiditis associated with cauda equina syndrome has not been reported in Korea until now. The authors experienced cauda equina syndrome caused by adhesive arachnoiditis of the lumbar spine with satisfactory results following decompression. We report this case with a review of the relevant literature.

A Rare Cause of Thoracic Spinal Cord Compression by Multiple Large Tarlov Cysts / 대한신경손상학회지

Spinal extradural arachnoid cyst (SEAC) is a rare cause of spinal cord compression. Bifocal location of thoracic and sacral SEACs is rarely reported in the literature. We report a case of thoracic spinal cord compression by SEAC associated with asymptomatic multiple sacral Tarlov cysts (TC). The surgical management and postoperative outcome of the patient are discussed. A 34-year-old woman was referred to the hospital for acute thoracic pain with a history of chronic long-standing back pain. She complained of walking difficulties. Neurological examination demonstrated incomplete spastic paraplegia with sensory level in T9. Magnetic resonance imaging revealed a large cystic formation from T7-11 and at the level of the sacrum. We performed laminectomies at the level of interest from T7-11. The cysts were dissected from the underlying dura after removal of the cerebrospinal fluid. We found nerve tissue in the cysts. We excised the cyst and preserved the nerve roots. Subsequently, a duraplasty was performed with autologous grafts from the lumbar fascia. The condition of the patient improved after surgery and he was recovering well at follow-up. Although the surgical treatment of TC is controversial, especially at the sacral lumbar level, decompression at the dorsal level in this case is indisputable.

Primary extracranial meningioma presenting as a forehead mass

Meningioma originates from arachnoid cap cells and is the second most common intracranial tumor; however, it can also be found in an extracranial location. A very rare primary extracranial meningioma without the presence of an intracranial component has also been reported. Primary extracranial meningiomas have been found in the skin, scalp, middle ear, and nasal cavity. A computerized tomography or magnetic resonance imaging scan is necessary to determine the presence or absence of an intracranial meningioma, and a biopsy is essential for diagnosis. We report a case of primary extracranial meningioma located in the forehead skin of a 51-year-old male.

Hypomania in Bobble-Head Doll Syndrome: A Case Report of Surgically Treated Stereotypy and Hypomania

A 22-year-old man was admitted with gradually aggravating stereotypic head movement with hypomania. Brain magnetic resonance imaging showed a large suprasellar arachnoid cyst extending into the third ventricle, with obstructive hydrocephalus, characteristic of bobble-head doll syndrome. Endoscopic fenestration of the suprasellar arachnoid cyst was performed. Stereotypic head movement stopped immediately after surgery and hypomanic symptoms gradually improved within a month. During 4 years of follow-up observation without medication, neuropsychiatric symptoms did not relapse. We report our experience of surgically treating stereotypy and hypomania in a case of bobble-head doll syndrome and discuss the possible neuropsychiatric mechanisms of this rare disease.

Arachnoid Granulations Mimicking Multiple Osteolytic Bone Lesions in the Occipital Bone

We report a rare case of arachnoid granulations mimicking multiple osteolytic bone lesions. A 66-year-old woman was admitted to a local clinic for a regular checkup. Upon admission, brain CT showed multiple osteolytic lesions in the occipital bone. These needed to be differentiated from multiple osteolytic bone tumor. Subsequent brain MRI revealed that the osteolytic lesions were isointense to cerebrospinal fluid, hyperintense on T2-weighted image, hypointense on T1-weighted image, and with subtle capsules around the osteolytic lesions that were visible after gadolinium injection. A bone scan revealed no radiotracer uptake. The lesions were in both the transverse sinuses and the torcular herophili. With typical radiological appearances of the lesions, the osteolytic lesions were diagnosed as multiple arachnoid granulations. No further treatment was planned. A 1-year follow-up brain CT scan revealed no change. We should consider the possibility of arachnoid granulations when multiple osteolytic lesions are observed in the occipital bone.

Subarachnoid Hemorrhage with Subdural Hematoma due to Ruptured De Novo Aneurysm after Aneurysmal Clipping via Pterional Approach: An Autopsy Case / 대한법의학회지

Subdural hematoma (SDH) due to spontaneous rupture of intracranial aneurysm rarely occurs. The prevalence of subarachnoid hemorrhage (SAH) with SDH is approximately 0.5%–10.3% of all aneurysmal SAH. We report a case of aneurysmal rupture with SDH and SAH due to arachnoid defect after aneurysm clipping. The decedent was a 51-year-old woman who underwent brain surgery for SAH a few years ago. Two days before she died, she had nausea and sentinel headache. She was alive in the morning and was found dead at 6 PM. Injuries in the external surface were not found. A fresh SDH, measured approximately 90 mL, was found in the right hemisphere. SAH was diffusely distributed at the base of the brain and the right sylvian fissure. Two aneurysmal clippings were found in the anterior communicating artery and right internal carotid artery. A ruptured de novo aneurysm was also found in the right proximal middle cerebral artery. An uncal herniation was also observed. The cause of death was SAH with SDH due to de novo intracranial aneurysm. The tearing caused by the adhesion between the aneurysm and arachnoid, high blood pressure, and massive bleeding has been thought to be the causative mechanism of aneurysmal SAH with SDH. However, in this case, the arachnoid defect was caused by aneurysmal clipping through pterional approach. This defect served as the passage between the subarachnoid and subdural spaces. The autopsy for recurrent intracranial aneurysm will increase according to the extending life expectancy of patients after aneurysmal clipping.

Modified Arachnoid Plasty Reduces Chronic Subdural Hematoma after Unruptured Aneurysm Clipping : Technical Note

OBJECTIVE: Chronic subdural hematoma (CSDH) is a rare complication of unruptured intracranial aneurysm (UIA) clipping surgery. To prevent postoperative CSDH by reducing subdural fluid collection, we applied the modified arachnoid plasty (MAP) during the UIA clipping surgery to seal the dissected arachnoid plane. METHODS: This retrospective study included 286 patients enrolled from July 2012 to May 2015. We performed arachnoid plasty in all patients, with MAP used after June 17, 2014. Patients were divided into two groups (non-MAP vs. MAP), and by using uni- and multivariate analyses, baseline characteristics, and relationships with postoperative CSDH between the two groups were analyzed. The degree of preoperative brain atrophy was estimated using the bicaudate ratio (BCR) index. RESULTS: Ten patients (3.5%) among 286 patients had postoperative CSDH after clipping. Nine (3.1%) were in the non-MAP group, and one (0.9%) was in the MAP group. The higher BCR index showed statistical significance with occurrence of postoperative CSDH in both uni- (p=0.018) and multivariate (p=0.012; odds ratio [OR], 8.547; 95% confidence interval [CI], 1.616–45.455) analyses. MAP was associated with a lower risk of postoperative CSDH (p=0.022; OR, 0.068; 95% CI, 0.007–0.683). CONCLUSION: This study shows that the degree of preoperative brain atrophy is associated with an increased occurrence of CSDH after clipping and that MAP could help reduce the risk of postoperative CSDH after unruptured aneurysm clipping via a lateral supraorbital approach.

Modified Arachnoid Plasty Reduces Chronic Subdural Hematoma after Unruptured Aneurysm Clipping : Technical Note

OBJECTIVE: Chronic subdural hematoma (CSDH) is a rare complication of unruptured intracranial aneurysm (UIA) clipping surgery. To prevent postoperative CSDH by reducing subdural fluid collection, we applied the modified arachnoid plasty (MAP) during the UIA clipping surgery to seal the dissected arachnoid plane.METHODS: This retrospective study included 286 patients enrolled from July 2012 to May 2015. We performed arachnoid plasty in all patients, with MAP used after June 17, 2014. Patients were divided into two groups (non-MAP vs. MAP), and by using uni- and multivariate analyses, baseline characteristics, and relationships with postoperative CSDH between the two groups were analyzed. The degree of preoperative brain atrophy was estimated using the bicaudate ratio (BCR) index.RESULTS: Ten patients (3.5%) among 286 patients had postoperative CSDH after clipping. Nine (3.1%) were in the non-MAP group, and one (0.9%) was in the MAP group. The higher BCR index showed statistical significance with occurrence of postoperative CSDH in both uni- (p=0.018) and multivariate (p=0.012; odds ratio [OR], 8.547; 95% confidence interval [CI], 1.616–45.455) analyses. MAP was associated with a lower risk of postoperative CSDH (p=0.022; OR, 0.068; 95% CI, 0.007–0.683).CONCLUSION: This study shows that the degree of preoperative brain atrophy is associated with an increased occurrence of CSDH after clipping and that MAP could help reduce the risk of postoperative CSDH after unruptured aneurysm clipping via a lateral supraorbital approach.

A Case of Endoscopic Transethmoidal Drainage of Subdural Abscess Originated from Dental Infection / 대한이비인후과학회지

Subdural empyema is fulminating purulent infection that develops between the dura and the arachnoid membranes. It is rare but one of the most immediate of neurosurgical emergencies, hence the importance of proper diagnosis and early intervention. Most subdural empyema complicated in the ENT field originate from sinonasal or otologic infections rather than dental infections. In our case of 64-years-old male, who was admitted with headache and fever, the diagnosed subdural empyema originated from odontogenic parapharyngeal abscess. We report that the case was successfully managed after urgent surgical drainage by endoscopic transethmoidal approach with long term intravenous antibiotics.

Extra-Axial and Clear Cell Type Ependymoma, Mimicking a Convexity Meningioma

A 33-year-old woman presented with tingling and paresthesia on left extremity for 2 months. Magnetic resonance imaging revealed that the tumor was iso- and hypo-intensity on T1-weighted image, mixed iso- and high-signal intensity on T2-weighted images and heterogeneously enhanced with rim enhancement. Neither arachnoid cleft nor dural tail was certain but mass was located extra-axially so meningioma was suspected. During operation, tumor wasn't attached to dura at all but arachnoid attachment was seen. Pathologically, clear cell type ependymoma was confirmed. Details of diagnosis and treatment of this tumor is described.

Idiopathic Cervicosyringomyelia in 2 year-old Boy / 대한소아신경학회지

Syringomyelia is a disorder in which a cavity has formed within the spinal cord. Idiopathic syringomyelia is not associated with identifiable causes such as Chari type 1 malformation, spinal cord tumor, vascular malformation, tethered cord, arachnoiditis, hydrocephalus, or previous spinal surgery. The main neurologic symptoms of idiopathic syringomyelia are toe-walking, constipation, incontinence, abnormal reflexes, and lower extremity weakness. Patients may present with various symptoms such as scoliosis, cutaneous markers, pain in the lower extremities or back, or may be asymptomatic. Herein, we report a young child with idiopathic syringomyelia presenting with subtle neck pain. A 23-month-old boy visited the neurologic clinic after 3 months of right occipital area neck pain. He had no history of trauma or central nervous system infection, and neurologic examination results were normal except for right posterior neck hyperesthesia. Brain and spinal magnetic resonance imaging showed an ovoid intramedullary cystic lesion (9.7×5.0×4.7 mm) at C6/7 of the spinal cord. There was no evidence of Chiari malformation or other lesions that can be primary pathologies of syringomyelia. Electromyogram/nerve conduction velocity results were normal. The subject was diagnosed as idiopathic syringomyelia. His symptoms and neurologic/radiologic indications showed no change at a 1-year follow-up. Idiopathic syringomyelia symptoms are varied and may be overlooked by physicians. Pediatricians may consider syringomyelia if patients complain about persistent sensory abnormality. All patients who present with syringomyelia should undergo detailed neuroimaging of the entire neuraxis to elucidate the proximate cause of the lesion.

Intradural Extramedullary Capillary Hemangioma In the Upper Thoracic Spine with Simultaneous Extensive Arachnoiditis

Capillary hemangiomas are common benign vascular tumors on skin and soft tissues, but developing as an intradural and extramedullary (IDEM) tumor in spine is extremely rare. In this report, we present IDEM tumor compressing thoracic cord in T2–3 level with extensive arachnoiditis below the tumor level in a 60-year-old man. The lesion was removed and histological diagnosis was capillary hemangioma. Prompt diagnosis and resection are important to avoid neurological deterioration from acute hemorrhagic condition. Simultaneous arachnoiditis may be originated from old subarachnoid hemorrhage associated tumor before diagnosis, and we suggest it as a helpful diagnostic feature to suspect vascular tumors such as capillary hemangioma.

Hemorrhagic Rupture of Arachnoid Cyst into the Intradural Space

A 7-year-old boy, diagnosed with an arachnoid cyst and subdural effusion on initial MRI, was admitted with left limb weakness and no history of head trauma. A subsequent follow-up MRI showed different stages of hematoma within multilayered enhancing membranes and in the arachnoid cyst, which was separated by the cerebrospinal fluid cleft. Craniotomy and fenestration of the cyst wall and hematoma removal were performed. The patient was diagnosed as a having a hemorrhagic rupture of an arachnoid cyst into the intradural space, probably via some one-way valve-like defect, based on the MRI and surgical findings. The MRI features and possible mechanism of this rare disease are discussed within the literature review.

Giant Arachnoid Granulations in Headache Mimicking Migraine with Aura

Giant arachnoid granulations have been reported to be associated with headaches, which can be acute or chronic in presentation. In some cases, idiopathic intracranial hypertension, previously called pseudotumor cerebri, may occur. The pathophysiology of these enlarged structures seen as filling defects on imaging is not clearly defined, although they are presumed to cause symptoms such as headache via pressure resulting from secondary venous sinus obstruction. We present a unique presentation of secondary headache in a 39-year-old man with no prior history of headaches found to have giant arachnoid granulations, presenting as migraine with aura.